Landau-Kleffner syndrome: long-term follow-up.
OBJECTIVE
Landau-Kleffner syndrome (LKS) is a rare entity characterized by epilepsy and aphasia. It occurs in previously normal children, usually between three and seven years of age. The long-term outcome of LKS is not completely clear. The aim of this study is to verify the long-term follow-up of a group of patients with LKS, focusing on clinical and electroencephalographic (EEG) aspects, and quality of life.


METHODS
This was a transversal study. Between November 2006 and April 2007 seven patients with previous diagnosis of LKS were interviewed. They had had a follow-up of three to 16 years after their disease onset. They were all males between the ages of eight and 27 years old. All patients had normal MRI. Parents and/or patients were interviewed by one of the authors using a structured questionnaire. The Vineland Adaptive Behavior Scales, the Conner's Rating Scales-Revised, and Short-Form Health Survey (SF 36) were used. Each patient had a prolonged interictal EEG recording. All patients had normal MRI.


RESULTS
The present investigation revealed that two patients still have seizures several years after epilepsy onset. One patient had total and three others had partial remission of language disturbance, while three patients still have aphasia and verbal auditory agnosia. With respect to quality of life, only one of our patients has a normal life at present. The remaining six patients with some sort of limitation consider the aphasia/agnosia to be the main difficulty in their lives. Five patients have normal EEGs.


CONCLUSIONS
The long-term follow-up of patients with LKS shows that epilepsy and EEG abnormalities do not always disappear. Language disturbances tend to persist in most patients. The age of onset of language dysfunction does not seem to correlate with the prognosis for recovery of language function. Patients with LKS have an overall poor quality of life, mostly due to language difficulties.
Duran
Marcos H C
MH
Department of Neurology, FCM, State University of Campinas (Unicamp), P.O. Box 6111, 13083-970 Campinas, SP, Brazil.
Guimarães
Catarina A
CA
Medeiros
Lívia L
LL
Guerreiro
Marilisa M
MM
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000377:Agnosia / Q000209:etiology / Q000503:physiopathology; D001037:Aphasia / Q000209:etiology / Q000503:physiopathology; D002648:Child; D004569:Electroencephalography / Q000379:methods; D004827:Epilepsy / Q000209:etiology / Q000503:physiopathology*; D005500:Follow-Up Studies; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D007806:Language Disorders / Q000209:etiology / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D011788:Quality of Life; D011795:Surveys and Questionnaires; D013997:Time Factors; D055815:Young Adult